Kulinski Joseph M, Eisch Robin, Young Michael L, Rampertaap Shakuntala, Stoddard Jennifer, Monsale Joseph, Romito Kimberly, Lyons Jonathan J, Rosenzweig Sergio D, Metcalfe Dean D, Komarow Hirsh D
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
Clinical Research Directorate, Frederick National Laboratory for Cancer Research sponsored by the National Cancer Institute, Frederick, Md.
J Allergy Clin Immunol Pract. 2020 Jan;8(1):292-301.e2. doi: 10.1016/j.jaip.2019.07.004. Epub 2019 Jul 15.
Mastocytosis is a clonal mast cell disorder associated with elevated mast cell mediators, which themselves have been reported to affect lymphocyte function. However, the impact of an expanded mast cell compartment on lymphocyte subpopulations, and their correlation with clinical phenotypes in patients with indolent systemic mastocytosis (ISM), has not been explored.
To examine the immunophenotype of circulating lymphocytes in patients with ISM compared with healthy adult controls and examine relationships with aspects of clinical disease.
We examined lymphocyte subsets in 20 adult patients with ISM and 40 healthy adult volunteers by multiparameter flow cytometry. Results were correlated with clinical characteristics.
Patients with ISM exhibited a significantly lower median frequency and absolute cell count of both circulating CD8 T cells and natural killer cells accompanying a significantly increased ratio of CD4/CD8 T cells when compared with healthy volunteers. Stratification of our ISM patient cohort according to clinical manifestations revealed that CD19CD21CD38 B cells were significantly higher in patients with a history of autoimmune disease and counts of terminally differentiated CD4 T cells were significantly higher in patients with osteoporosis or osteopenia.
Several circulating lymphocyte subpopulations in patients with ISM were significantly different when compared with healthy controls; in specific lymphocyte subsets, this lymphocyte skewing correlated with clinical observations including osteoporosis and autoimmune disease. These data suggest the need for further studies on abnormalities in lymphocyte subsets and the attendant clinical consequences in both mast cell proliferative and activation disorders.
肥大细胞增多症是一种与肥大细胞介质升高相关的克隆性肥大细胞疾病，据报道这些介质本身会影响淋巴细胞功能。然而，肥大细胞数量增加对惰性系统性肥大细胞增多症（ISM）患者淋巴细胞亚群的影响及其与临床表型的相关性尚未得到探讨。
比较ISM患者与健康成年对照者循环淋巴细胞的免疫表型，并研究其与临床疾病各方面的关系。
我们通过多参数流式细胞术检测了20例成年ISM患者和40名健康成年志愿者的淋巴细胞亚群。结果与临床特征相关联。
与健康志愿者相比，ISM患者循环CD8 T细胞和自然杀伤细胞的中位频率和绝对细胞计数显著降低，同时CD4/CD8 T细胞比值显著升高。根据临床表现对我们的ISM患者队列进行分层显示，有自身免疫疾病史的患者中CD19CD21CD38 B细胞显著增多，而骨质疏松或骨质减少患者的终末分化CD4 T细胞计数显著升高。
与健康对照相比，ISM患者的几种循环淋巴细胞亚群存在显著差异；在特定淋巴细胞亚群中，这种淋巴细胞失衡与包括骨质疏松和自身免疫疾病在内的临床观察结果相关。这些数据表明需要进一步研究肥大细胞增殖和激活障碍中淋巴细胞亚群异常及其伴随的临床后果。
